2. The normal sensori-motor nerve conduction with absent F-waves and tendon jerks of a moderately de-saturated semiconscious patient in the intensive care unit may be seen in: a. A classical pattern of critical illness myopathy involving both proximal and distal muscles b. Post-synaptic neuromuscular disorder c. The early stages of motor neuron disease (MND) (axonal loss) before the period of W...

Amyotrophic lateral sclerosis (ALS) is a devastating paralytic disorder caused by motor neuron degeneration. A subgroup of familial cases arises from mutations in the gene encoding cytosolic superoxide dismutase (SOD1). This review considers insight now being gained into ALS pathogenesis from the study of mutant SOD1 protein and its possible mechanisms of adverse effect on nerve cells. Also dis...

Abstract Although the term of amyotrophic lateral sclerosis (ALS) is often used interchangeably with motor neuron disease, ALS universally accepted as a multisystem disorder. Cognitive impairment an acknowledged feature ALS, affecting language, memory and behaviour, apathy considered to be prevalent behavioural alteration in ALS. It can divided three subtypes: executive, emotional initiation ap...

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative system disorder affecting both upper and lower motor neurons. Despite supportive electrophysiological investigations, the involvement of the upper motor neuron is often difficult to assess at an early stage of disease. Diffusion tensor MRI provides an estimate of the orientation of fibre bundles in white matter on the basis ...

brown-vialetto-van laere syndrome (bvvls) is a rare neurological disorder of unknown etiology considered to be a form of motor neuron diseases. this syndrome is characterized by bilateral deafness and involvement of lower cranial nerves, especially 7th-12th. umn signs are less frequent. until 2007, only fifty eight cases were reported. half of the reported cases were sporadic. in the remaining ...

Spinal muscular atrophy (SMA) is a common ( 1:6400) autosomal recessive neuromuscular disorder caused by a paucity of the survival of motor neuron (SMN) protein. Although widely recognized to cause selective spinal motor neuron loss when deficient, the precise cellular site of action of the SMN protein in SMA remains unclear. In this study we sought to determine the consequences of selectively ...

42 Amyotrophic lateral sclerosis (ALS) is a devastating paralytic disorder caused by dysfunction 43 and degeneration of motor neurons starting in adulthood. Recent studies using cell or animal 44 models document that astrocytes expressing disease-causing mutations of human 45 superoxide dismutase 1 (hSOD1) contribute to the pathogenesis of ALS by releasing a 46 neurotoxic factor(s). Neither the...

background: the failure of regeneration after spinal cord injury (sci) has been attributed to axonal demyelination and neuronal death. cellular replacement and white matter regeneration are both necessary for sci repair. in this study, we evaluated the co-transplantation of olfactory ensheathing cells (oec) and embryonic stem (es) cell-derived motor neurons (esmn) on contused sci. methods: oec ...